Cardiomyopathy

Cardiomyopathy refers to heart muscle disease. The damaged heart does not effectively pump blood. The disease usually progresses to the point where patients develop life-threatening heart failure.

There are three main types of Cardiomyopathy :

Dilated – Damaged heart muscles lead to an enlarged, floppy heart. The heart stretches as it tries to compensate for weakened pumping ability.

Hypertrophic – Heart muscle fibers enlarge abnormally. The heart wall thickens, leaving less space for blood in the chambers. Since the heart does not relax correctly between beats, less blood fills the chamber and is pumped from the heart.

Restrictive – Portions of the heart wall become rigid and lose their extensibility. Thickening often occurs due to abnormal tissue invading the heart.

Causes

In many cases, the exact cause is not known. Possible causes include:

Dilated – The cause of the initial damage is often not found but may include:
  • Chronic exposure to toxins, including alcohol, metals, and drugs
  • Infections (rare)
  • Diseases of the blood vessels, including the coronary arteries, which feed the heart
  • A rare complication of pregnancy or childbirth (probably immune related)
  • Transplant rejection
Hypertrophic
  • Congenital (abnormal thickening of heart muscle at birth)
  • Long-standing hypertension
Restrictive – usually related to another condition, such as:
  • Amyloidosis – protein fibers collect in the heart muscle
  • Sarcoidosis – small inflammatory masses (granulomas) form in many organs
  • Hemochromatosis – too much iron in the body

Risk Factors

A risk factor is something that increases your chance of getting a disease or condition.
  • Family members with Cardiomyopathy
  • Alcoholism
  • Obesity
  • Diabetes
  • Hypertension
  • Coronary artery disease
  • Certain drugs

Symptoms

Symptoms vary, depending on the type of Cardiomyopathy and its severity. Patients with hypertrophic Cardiomyopathy often do not notice any symptoms. Sudden cardiac arrest may be the first indication of the condition. In dilated Cardiomyopathy , it may take years for symptoms to develop. Blood clots may form due to the abnormal pooling of blood in the heart. If a clot moves to another part of the body (embolism), symptoms associated with that organ (the brain, for example) may be the first sign of the heart disease.

Cardiomyopathy ultimately leads to heart failure and the following symptoms:
  • Fatigue
  • Weakness
  • Shortness of breath, often worse when lying down and with exertion
  • Cough
  • Swelling in feet or legs
  • Chest pain
  • Irregular heart rhythm

Diagnosis

The doctor will:
  • Ask about your symptoms and medical history
  • Perform a physical exam
  • Listen to your heart with a stethoscope (Cardiomyopathies often produce heart murmurs and other abnormal heart sounds.)
Tests may include:

Chest X-ray – to look for heart enlargement

Electrocardiogram – a test that records the heart's activity by measuring electrical currents through the heart muscle

Echocardiogram– a test that uses high-frequency sound waves (ultrasound) to examine the size, shape, and motion of the heart

Blood Tests – to check for damage to the heart and other organs, and possibly the underlying cause(s) of the Cardiomyopathies

Cardiac Catheterization –a tube-like instrument inserted into the heart through a vein or artery (usually in the arm or leg) to detect problems with the heart and its blood supply

Heart Biopsy – removal of a sample of heart tissue for testing

Treatment

No treatment, short of a heart transplant, can reverse the damage already done to the heart muscle. Treatment aims to relieve symptoms and prevent further damage. If a treatable underlying cause is found, treatment will be started to address the problem.

Lifestyle Modification

Changes aim to eliminate anything that contributes to the disease or worsens symptoms:
  • Avoid alcohol.
  • Lose weight, if you are overweight.
  • Eat a low-fat diet to minimize the risk and extent of coronary artery disease.
  • Limit salt intake to decrease fluid retention.
  • Follow your doctor's advice for exercise. You may need to limit physical activity.
Medications

Medications include:
  • Diuretics – to eliminate extra fluid
  • ACE inhibitors – to help relax blood vessels, lower blood pressure and decrease the heart's workload
  • Digitalis – to slow and regulate the heart rate, and modestly increase its force of contractions
  • Calcium channel blockers – to lower blood pressure and relax the heart
  • Beta blockers – to slow the heart and limit disease progression
  • Anti-arrythmia agents – to prevent irregular heart rhythms
  • Immune system suppressants – including steroids (depending on underlying cause)
Surgery

Surgical options include:
  • A pacemaker may be implanted to change the heart rate and pattern.
  • For patients with hypertrophic disease, doctors may remove part of the thickened wall separating the heart's chambers. Surgery may be needed to replace a heart valve.
  • A heart transplant may be possible for otherwise healthy patients who do not respond to medical treatment. Candidates often wait a long time for a new heart. Those waiting may temporarily receive a ventricular assist device, which is a mechanical pump that assumes some or most of the heart's pumping function.

Prevention

Aggressively treating hypertension, coronary artery diseases, and their risk factors is the best way to prevent most cases of cardiomyopathy. Other less common causes, however, are not preventable. People with a family history of the disease should ask the doctor about screening tests, especially before starting an intense exercise program.